Mid-Brain Cavernoma (Cavernous Malformation)

Contributor:

Dr. Daryl Fourney

Consultants:

Dr. Gary Steinberg
Dr. Mike Tymianski
Dr. Fred Gentili
Dr. Chris Ekong

Case 29

Age: 35

Sex: Female

History: December 2004. 35 yo female otherwise healthy presented 1 week ago with headache/confusion after a night of drinking. On exam has complete L III palsy, subtle R III palsy (mild ptosis, poor accommodation), mild R dysmetria. Over the last week, confusion has almost completely resolved.

December 22, 2004 - Dr. Daryl Fourney, Neurosurgeon, Saskatoon

We originally thought this was a thrombosed basilar tip aneurysm, but the angio is completely normal and the MRI is more in keeping with a cavernous malformation.

We are waiting for the blood products to dissipate to confirm the Dx on follow-up imaging.

Assuming this is a cavernoma, here are some questions:

- What is the rate of rehemorrhage and the prognosis?

- Would anybody operate on this, our would you wait for a rehemorrhage?

- What role, if any, does radiosurgery play?

Daryl

December 22, 2004 - Dr. Chris Ekong, Neurosurgeon, Regina

To: Neuro Vascular Medi-Fax Rounds Group:

Daryl Fourney, a neurosurgeon in Saskatoon wonders what you would do for this lady.

Chris

December 23, 2004 - Dr. Chris Ekong, Neurosurgeon, Regina

Daryl, I think this case is similar to Dr. Steinberg's case of November 2003 Internet Rounds. The following movie shows his case, and what he and others like Mike Schwartz Mike Tymianski, Ian Fleetwood, etc. suggested.

December 29, 2004 - Dr. Mike Tymianski, Neurosurgeon, Toronto

Hello Chris Ekong et al.

My answers are below:

- What is the rate of rehemorrhage and the prognosis?

According to the best published evidence (Porter et al., J Neurosurg. 1997 Aug;87(2):190-7. ), this patient has a neurological event rate of 10.6% per year. Of the individuals who suffer a hemorrhage, about 1/3 improve completely, 1/3 improve partially, and 1/3 sustain a persistent deficit.

- Would anybody operate on this, or would you wait for a rehemorrhage?

The issue is less about a re-hemorrhage, and more about what we'd be operating on. Given the MRI''s posted on the web site, I cannot actually discern the cavernoma - I only see the hemorrhage, and the developmental venous anomaly that's enhancing with gadolinium and which, presumably, is associated with the cavernoma. It might be helpful to study the entire series of images to see whether the cavernoma can be seen.

I suspect that the cavernoma is in the region of the DVA.

Another problem that I have encountered is that sometimes, the hemorrhage is that which makes the lesion appear to be at the pial surface. However, unless surgery is performed within a short period of time (at most 2-3 weeks), the hematoma will resorb to the extent that the lesion may be less accessible (see the attached pictures of a 28 Yr male with a midbrain cavernoma at the time of hemorrhage and again at the time of surgery, 4 weeks later).

Therefore, I would not plan surgery on this Case 29 without 1. Seeing the full original MRI and 2. Repeating it now that the patient has improved.

If, on the latest scan, I can:

1. See the Cavernoma and

2. See it against a pial surface,

then I would discuss the pros and cons of surgery with the patient.

Pros: Cure from a 10.6% per year event rate in this young person. Notably, this leasion is close to the aqueduct, and I have seen a death due to obstructive hydro from a cavernoma in this location.

Cons: 30-40% chance of temporary worsening after surgery. 10% chance of permanent neurological deficits, including worsening of her cranial nerve palsies.

- What role, if any, does radiosurgery play?

It is our feeling that there is no useful role for radiosurgery in the treatment of these lesions. I believe that the discussion in the "Steinberg" commentary appended to Case 29 covers this quite nicely.

I hope this is helpful.

Happy Holidays to all.

Mike Tymianski

December 29, 2004 - Dr. Fred Gentili, Neurosurgeon, Toronto

Dear Chris and all,

Michael Tymianski has addressed the main issues nicely.

Firstly I do not think that anything needs to be done urgently. I agree with

a follow-up MRI scan or scans to better delineate the size and exact location of the lesion. I would allow the patient to achieve maximal neurological recovery.

Surgery would be feasible only if the lesion reaches the pial surface. The approach will on depend on where on the axis of the brain-stem it reaches the pial surface.

While statistics count I have two patients with lesions in similar areas that are 15 years and 8 years after their initial bleeds with no further events. Virtually all patients are initially worse after surgery but most do improve.

Unless the patient was desperately seeking treatment I would likely await a second event in this patient prior to considering surgery. Based on the literature I don't think that radiosurgery has any role to play in these lesions.

Regards,

Fred

December 29, 2004 - Dr. Gary Steinberg, Neurosurgeon, Stanford

Daryl, Chris, and colleagues:

I would not operate acutely in this patient. The case I presented at the Internet Rounds in Toronto, was of a young woman who had clinically bled 3 times, and the malformation extended to the top of the IIIrd ventricle, so could be accessed through the foramen of Monro. This current hemorrhage is located in the lower portion of the III ventricle and more difficult to reach from above.

I would repeat the MR in about 2 months. As the blood resorbs, it will be easier to visualize the actual malformation. Since the hemorrhage is more anterior, the CM may be closer to the anterior surface of the midbrain. If this were true and if surgery were indicated, a trans-Sylvian or

orbitozygomatic approach would be preferable. I have used this with success in the past.

I'm not sure I would operate on this patient after only 1 hemorrhage, although I suspect the cumulative lifetime risk of rehemorrhage is relatively high.

No role for radiosurgery.

Thanks,

Gary

December 30, 2004 - Dr. Chris Ekong, Neurosurgeon, Regina

Summary:

Daryl, the experts have spoken.

1. Risk of hemorrhage: high.

2. Surgery? Not indicated at this time. Repeat MRI in 2 months after the blood would have resorbed. Decision for surgery would depend on if lesion is close to surface. Generally speaking no surgery with one hemorrhage alone.

3. No Radiosurgery

Friday, January 7, 2005 - Dr. Max Findlay, Neurosurgeon, Edmonton

Chris,

Here is my 2 cents worth of comments on case 29, Daryl's midbrain hematoma presumed cavernous malformation. I did not want to sound monotonous since so many smarter people had already said "don't operate". I should tell you though, about a very similar case in the tectum of a young woman where we saw a quick neurological recovery and of course did not explore, and 2 years later the malformation was almost undetectable on the MRI. She is fine now 8 years and 3 kids later. I know because the obstetricians call me everytime she gets pregnant.

Chris--hope this finds you well.

Max

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